Publications by authors named "Eileen M Lynch"

Article Synopsis
  • TDP-43 is an RNA binding protein that forms aggregates in the central nervous system and is notably present in certain neurodegenerative diseases and inclusion body myopathy, a type of muscle disease.
  • Researchers developed a mouse model that shows muscle weakness associated with TDP-43 accumulation, which indicates a prion-like spread of the protein possibly affecting muscle tissues.
  • Human muscle biopsies from patients with various conditions, especially inclusion body myositis (IBM), contain TDP-43 aggregate seeds, suggesting a unique pathogenic role for TDP-43 in muscle diseases that wasn't fully recognized before.
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TAR DNA-binding protein 43 (TDP-43) is an RNA binding protein that accumulates as aggregates in the central nervous system of some neurodegenerative diseases. However, TDP-43 aggregation is also a sensitive and specific pathologic feature found in a family of degenerative muscle diseases termed inclusion body myopathy (IBM). TDP-43 aggregates from ALS and FTD brain lysates may serve as self-templating aggregate seeds and supporting a prion-like spread from cell to cell.

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Amyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disease in which patients gradually become paralyzed due to loss of motor function. Many genetically inheritable mutations have been linked to ALS; however, the majority of ALS patients are considered sporadic. Therefore, there is a need for a common therapy that is effective for all ALS patients.

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Recent advances in bioengineering have enabled cell culture systems that more closely mimic the native cellular environment. Here, we demonstrated that human induced pluripotent stem cell (iPSC)-derived myogenic progenitors formed highly-aligned myotubes and contracted when seeded on two-dimensional micropatterned platforms. The differentiated cells showed clear nuclear alignment and formed elongated myotubes dependent on the width of the micropatterned lanes.

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